低骨矿物质密度及其在没有疾病修饰治疗的情况下影响脊髓肌肉缩的因素:单中心横截面研究
Chuan Liu1, Dandan Yang2, Lekai Luo1
1Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education, West China Second University Hospital, Sichuan University, 20# Section 3 South Renmin Road, Chengdu, 610041, Sichuan, China.
BMC pediatrics
|October 11, 2024
概括
低骨矿物质密度 (BMD) 在没有治疗的情况下患有脊髓肌缩 (SMA) 的儿童中很常见. 年龄是一个关键因素,6.3岁或以上的儿童患低BMD的风险更高.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 神经肌肉疾病 神经肌肉疾病
- 骨的新陈代谢 骨的新陈代谢
背景情况:
- 脊椎肌肉缩 (SMA) 的儿童面临低骨矿物质密度 (BMD) 和骨脆弱性的风险增加.
- 这项研究侧重于小儿SMA患者没有接受疾病修饰治疗.
研究的目的:
- 用定量计算断层扫描 (QCT) 来评估SMA儿童的腰椎骨干骨质量.
- 为了确定影响儿童群体低BMD的因素.
主要方法:
- 收集了66名小儿SMA患者的人口统计,实验室,QCT和脊柱放射数据.
- 采用线性回归和ROC分析来探索BMD和影响因素之间的相关性.
主要成果:
- 在分析的SMA患者中,有28.8%的患者表现出低的BMD (Z-score ≤ -2.0).
- 骨质量Z-分数与年龄有显著的相关性 (p=0.001).
- 年龄≥6.3岁预测低BMD,敏感度为68.4%,特异性为68.1%.
结论:
- 在缺乏疾病修饰治疗的SMA儿童中,低的BMD非常普遍.
- 对所有类型的SMA来说,定期的BMD监测至关重要,特别是对于年龄在6.3岁及以上的人来说.
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